Share this story

Dr James Galvin

Special reports

Author: Geoffrey ChangPublished: 19 November 2015

Prep: Cook: Serves:

Amid revelations that Robin Williams also suffered from Lewy body dementia, as well as Parkinson’s disease, before his death last year, we shed light on the little-known dementia, often misdiagnosed as Parkinson’s and described by experts as “the most common disease you have never heard of”

“Lewy body dementia killed Robin – it’s what took his life,” said Susan Schneider, the wife of Robin Williams, speaking publicly for the first time since her husband’s death.

Not heard of Lewy body dementia or LBD before? You’re not alone. According to Dr James Galvin, a neurology and psychiatry professor at Florida Atlantic University: “It’s the most common disease you have never heard of.”

Sometimes referred to as ‘Parkinson’s with Lewy bodies’ or ‘Dementia with Lewy bodies’, it’s the second-most common dementia after Alzheimer’s, affecting memory, movement and motor control, while also causing visual hallucinations and severe psychiatric symptoms.

Disguised dementia

It’s a disease described by Jacqueline Cannon of the Lewy Body Society as “the worst bits of Alzheimer’s and the worst bits of Parkinson’s put together”. The symptomatic similarities that LBD shares with these diseases means it is often initially misdiagnosed as one or the other. It is, however, considered far more brutal and viciously progressive.

While the patient is living, the diagnosis process is not conclusive, with tests that are only able to rule out other conditions by deduction. Absolute diagnosis can only be confirmed after death if Lewy bodies are found in the brain at post-mortem examination.

Discovering LBD

While researching Parkinson’s disease in 1912, German neurologist Freiderich H Lewy (who was a colleague of Dr Alzheimer) discovered small deposits of the alpha-synuclein protein present in the brain cells of people with the condition. These proteins were later named Lewy bodies and are occur in both LBD and Parkinson’s.

The deposits develop inside some nerve cells (neurons) in the brain at the synapses, interrupting messaging and causing neurons to die. The patient could develop dementia if they are found in the cortex, or Parkinsonism if found in the brain stem.

“A sea monster with 50 tentacles of symptoms”

“This disease is a sea monster with 50 tentacles of symptoms that show when they want,” Schneider said of LBD. “We were living a nightmare.”

The long list of unpredictable symptoms includes: impaired visual perception and spatial awareness, delusions, hallucinations and loss of motor control.

One of the main differences to Alzheimer’s disease is that the patient is aware of the mental deterioration, that they are losing their mind. And the more aware they are of the situation, the more likely depression is to worsen.

“Robin was very aware that he was losing his mind and there was nothing he could do about it”

Schneider added: “Robin was very aware that he was losing his mind and there was nothing he could do about it.”

Jacqueline Cannon said of her father’s condition: “He always used to say to me, ‘I’m losing my mind’. We say to people that LBD is not just about memory. It’s about the other symptoms that go with it, especially the hallucinations.”

Professor Ian McKeith, president of the Lewy Body Society, believes there is cause for hope however. In a piece published by The Conversation, he wrote: “Therapeutic trials have been few and far between in LBD because of a combination of a lack of compounds to test, a pre-occupation with targeting Alzheimer’s and a reluctance of regulatory bodies to recognise LBD. All of these are now changing and LBD is increasingly viewed as a malleable and commercially-viable target.”

IN THE NEWS

Carefully selected news stories from the international Parkinson's community.

5 hours ago

Sniff test could detect Parkinson’s disease up to a decade earlier

A study has found that white adults with a poor sense of smell are almost five times more likely to develop Parkinson’s disease than those with a stronger smell sense. The research, published in the medical journal ‘Neurology’, found that there was not a statistically significant link between Parkinson’s and smell for black adults. Speculating on the reaction that people from different racial backgrounds had to the test, researchers said: “One possibility is that, compared to white participants, the etiology of olfactory dysfunction in black participants is more diverse and complex, and that Parkinson’s disease-related pathology is a relatively minor contributor.” The team emphasised that the findings should be interpreted with caution – and that further studies are needed before the smell tests can reach a clinical stage.

Spiral drawing test could detect early signs of Parkinson’s

Researchers from RMIT University, Australia, have developed a test that may be able to detect early Parkinson’s – before physical symptoms appear. During the test, participants draw a spiral using a tablet device, and computer software then measures their drawing speed and pen pressure to diagnose the condition. PhD researcher Poonam Zham led the study, published in ‘Frontiers of Neurology’, with the RMIT biomedical engineering research team. Working with Dandenong Neurology, the study involved 62 people diagnosed with Parkinson’s. Half had no visible symptoms and half ranged from mildly- to severely-affected. Professor Dinesh Kumar, chief investigator, said: “The customised software we’ve developed records how a person draws a spiral and analyses the data in real time. “With this tool we can tell whether someone has Parkinson’s disease and calculate the severity of their condition, with a 93% accuracy rate.” Image credit: RMIT University – Professor Dinesh Kumar and Poonam Zham

Asthma drug could halve chance of developing Parkinson’s

A recent study has found that a drug most commonly used for asthma may cut the chances of developing Parkinson’s. The research, carried out by Harvard Medical School, Massachusetts, US, found that those who inhaled salbutamol – a drug found in inhalers – were half as likely to develop the condition. In Parkinson’s disease, a protein named a-synuclein accumulates in various brain cells and can be fatal. To counter this, the research team grew human nerve cells and tested over 1000 medications, finding results to suggest salbutamol could cut the production of a-synuclein. Neurologist Anthony Lang, who works at the University of Toronto, Canada, said the results were “fascinating” and “had come out of the blue”. Despite some encouraging developments, Clemens Scherzer, who was a part of the research team, said clinical trials were “a few years off”.